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The Small-Molecule Flunarizine in Spinal Muscular Atrophy Patient Fibroblasts Impacts on the Gemin Components of the SMN Complex and TDP43, an RNA-Binding Protein Relevant to Motor Neuron Diseases

The motor neurodegenerative disease spinal muscular atrophy (SMA) is caused by alterations of the survival motor neuron 1 (SMN1) gene involved in RNA metabolism. Although the disease mechanisms are not completely elucidated, SMN protein deficiency leads to abnormal small nuclear ribonucleoproteins (...

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Detalles Bibliográficos
Autores principales: Sapaly, Delphine, Delers, Perrine, Coridon, Jennifer, Salman, Badih, Letourneur, Franck, Dumont, Florent, Lefebvre, Suzie
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Frontiers Media S.A. 2020
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7181958/
https://www.ncbi.nlm.nih.gov/pubmed/32363199
http://dx.doi.org/10.3389/fmolb.2020.00055